Brugada syndrome natural history, complications and prognosis
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Overview
The Brugada patient may develop atrial arrhythmias and abnormalities in atrial conduction, and these abnormalities are associated with inducibility of ventricular fibrillation.
Natural History
Brugada syndrome usually becomes apparent in adulthood, although signs and symptoms, including sudden death, can occur any time from early infancy to old age. The mean age of sudden death is approximately 40 years. This condition may explain some cases of sudden infant death syndrome (SIDS), which is a major cause of death in babies younger than one year. It is characterized by sudden and unexplained death, usually during sleep. Sudden unexplained nocturnal death syndrome (SUNDS) is a condition characterized by unexpected cardiac arrest in young adults, usually at night during sleep. This condition was originally described in Southeast Asian populations, where it is a major cause of death. Researchers have determined that SUNDS and Brugada syndrome are the same disorder.
Patients with Brugada syndrome frequently develop or are born with supraventricular tachycardias:[1]
- Supraventricular tachycardia: 20% of Brugada patients
- Atrial fibrillation: 10% - 20% of Brugada patients
- Atrioventricular (AV) nodal reentrant tachycardia
- Wolff-Parkinson-White syndrome[2]
Disturbances of atrial conduction and sinus node function have also been reported:
- Prolonged sinus node recovery time and sinoatrial conduction time [3]
- Slowed atrial conduction[4]
- Atrial standstill[4]
The appearance of atrial arrhythmias and impaired atrial conduction are remarkable in so far as these findings are associated with inducibility of ventricular fibrillation.[5]
In patients with an indication for an implantable cardioverter defibrillator (ICD), the incidence of atrial arrhythmias was 27% versus 13% in patients without an indication for an ICD (P<0.05), which suggests a more advanced disease process in patients with Brugada syndrome and spontaneous atrial arrhythmias. Inappropriate shocks from atrial arrhythmia episodes were observed in 14% of cases, highlighting the need for careful programming of the ICD.15
Complications
The following arrhythmias may occur in the patient with Brugada syndrome:
- Polymorphic VT resembling a rapid Torsade de Pointes (TdP)
- Monomorphic VT is observed infrequently
- VT/VF often terminates spontaneously in patients with the Brugada syndrome which may explain why patients wake up at night after episodes of agonal respiration caused by the arrhythmia.
Prognosis
Patients who are symptomatic with unexplained [[syncope], ventricular tachycardia or aborted sudden cardiac death may have a symptom recurrence risk of 2% to 10% per year. In these patients an AICD implant is advisable.
References
- ↑ Morita H, Kusano-Fukushima K, Nagase S, Fujimoto Y, Hisamatsu K, Fujio H, Haraoka K, Kobayashi M, Morita ST, Nakamura K, Emori T, Matsubara H, Hina K, Kita T, Fukatani M, Ohe T. Atrial fibrillation and atrial vulnerability in patients with Brugada syndrome. J Am Coll Cardiol. 2002; 40: 1437–1444.
- ↑ Eckardt L, Kirchhof P, Johna R, Haverkamp W, Breithardt G, Borggrefe M (2001). "Wolff-Parkinson-White syndrome associated with Brugada syndrome". Pacing and Clinical Electrophysiology : PACE. 24 (9 Pt 1): 1423–4. PMID 11584469. Unknown parameter
|month=ignored (help);|access-date=requires|url=(help) - ↑ Morita H, Fukushima-Kusano K, Nagase S, Miyaji K, Hiramatsu S, Banba K, Nishii N, Watanabe A, Kakishita M, Takenaka-Morita S, Nakamura K, Saito H, Emori T, Ohe T (2004). "Sinus node function in patients with Brugada-type ECG". Circulation Journal : Official Journal of the Japanese Circulation Society. 68 (5): 473–6. PMID 15118291. Retrieved 2012-10-13. Unknown parameter
|month=ignored (help) - ↑ 4.0 4.1 Takehara N, Makita N, Kawabe J, Sato N, Kawamura Y, Kitabatake A, Kikuchi K (2004). "A cardiac sodium channel mutation identified in Brugada syndrome associated with atrial standstill". Journal of Internal Medicine. 255 (1): 137–42. PMID 14687250. Retrieved 2012-10-13. Unknown parameter
|month=ignored (help) - ↑ Eur Heart J (2004) 25 (10): 879-884. doi: 10.1016/j.ehj.2004.01.004